Kawasaki disease

Dr. Sachin Soni
DNB Pediatrics
Indraprastha Apollo Hospital,
New Delhi

www.dnbpediatrics.com

What is it?


Medium vessel vasculitis presents as acute febrile
illness of childhood, characterized by coronary
arteries anomaly in 15-25% of affected
individuals


Epidemiology


Its common pediatric disorder with the annual incidence
range 60-150 per 100,000



Commonest pediatric vasculitis in children below 5
years of age(1-3)



Commonest vasculitic disorder amongst all ages


Etiology


Genetics



Autoantigen



Toxin mediated (STSS) super antigen mediated


Pathogenesis
Acute or subacute stages: Inflammatory infiltration of vascular wall, initially
polymorphonuclear cells thereafter by macrophages, lymphocytes
(primarily CD8+ T cells), and plasma cells
Eedema of endothelial and smooth muscle cells
IgA plasma cells are prominent in the inflammatory infiltrate
In most severely affected vessels, inflammation involves all three layers of the vascular wall
Destruction of the internal elastic lamina
Loss of structural integrity weakens the vessel wall
Dilation (ectasia) saccular or fusiform aneurysm formation
Thrombi in lumen and obstruct blood flow
vascular wall can become progressively fibrotic, marked intimal proliferation
Arterial stenosis or occlusion

EPIDEMIOLOGIC CASE DEFINITION
(CLASSIC CLINICAL CRITERIA)
Fever persisting for at least 5 days
 Presence of at least 4 principle feature
1- Changes in extremities:A- Acute:- Erythema of palm, soles and edema of hand and feet
B- Sub acute:- Periungul peeling of finger, toes in 2-3
polymorphus exanthema
2- B/L Bulbar conjunctival injection without exudates
3- Changes in lip and oral cavity:- Erythema, lip
cracking, strawberry tongue, diffuse injection of oral and
pharyngeal mucosa
4- Cervical lymphadenopathy (>1.5 cm diameter), usually
unilateral



Other Clinical Findings
Cardiovascular findings

Congestive heart failure, myocarditis,
pericarditis, valvular regurgitation,
Coronary artery abnormalities, Aneurysms
of medium-sized noncoronary arteries
Raynaud phenomenon, Peripheral
gangrene

Musculoskeletal system

Arthritis, arthralgias

Gastrointestinal tract

Diarrhea, vomiting, abdominal pain, Hepatic
dysfunction, Hydrops of gallbladder

Central nervous system

Extreme irritability, Sensoryneural hearing
loss, Aseptic meningitis,

Genitourinary system

Urethritis/meatitis

Clinical Menifestation


Percentages of cases


Laboratory finndings
Complete Hemogramme

Anemia
Leukocytosis with neutrophilia and
immature forms
Thrombocytosis after one week
Elevated erythrocyte sedimentation
rate (ESR)

Acute phase reactant

Elevated CRP

LFTs

Hypoalbuminemia, Elevated serum
transaminases, Elevated serum
gamma glutamyl
transpeptidase, Abnormal plasma
lipids

CSF

CSF Pleocytosis

others

Hyponatremia, Leukocytosis in
synovial fluid, sterile pyuria

Strawberry tongue


Congestion of bulbar
conjunctiva


Indurative edema of
the hands


Desquamation of the
fingers


Coronary Artery Aneurysm

Coronary angiogram demonstrating giant aneurysm of the left anterior
descending coronary artery (LAD) with obstruction and giant aneurysm of
the right coronary artery (RCA) with an area of severe narrowing in 6 yr old
boy


Two-dimensional echocardiography






Most useful test to monitor development of coronary
artery abnormalities
Brightness of the arterial walls and lack of normal
tapering of the vessels
Coronary artery dimensions, adjusted for body surface
area (BSA), are significantly increased in the first 5 wk
after presentation
BSA-adjusted coronary artery dimensions on baseline
echocardiography in the first 10 days of illness to be
good predictors of CAD


Aneurysm defined by Japanese Ministry of Health
classified as: Small (<5 mm internal diameter)
 Medium (5-8 mm internal diameter)
 Giant (>8 mm internal diameter)



Echocardiography performed at diagnosis and after 2-3 wk
of illness
If the results are normal, repeat study should be performed
6-8 wk after onset of illness


Diagnosis
Classic KD:- Diagnostic criteria

Fever for at least 4 days and at least four of five of
above principal characteristics of the illness

Atypical or incomplete KD:



Patients have persistent fever but fewer than four of
the five characteristics
In these patients, laboratory and echocardiographic
data can assist in the diagnosis
Incomplete cases are most frequent in infants,
who, unfortunately, also have the highest likelihood
of development of coronary artery abnormalities

Differential Diagnosis
VIRAL INFECTIONS

BACTERIAL INFECTIONS

Adenovirus,Enterovirus,Measles,EBV

Scarlet fever, Rocky Mountain spotted fever,
Leptospirosis, Bacterial cervical lymphadenitis

Systemic-onset juvenile idiopathic arthritis
RHEUMATOLOGIC DISEASE
OTHER

Toxic shock syndrome, Staphylococcal scalded skin
syndrome, Drug hypersensitivity reactions, StevensJohnson syndrome


TREATMENT
Acute phase:

Intravenous immunoglobulin 2 g/kg over 10-12 hr
AND



Aspirin 80-100 mg/kg/day divided every 6 hr orally
until patient is afebrile for at least 48 hr


CONVALESCENT STAGE



Aspirin 3-5 mg/kg once daily orally until 6-8 wk after
illness onset


LONG-TERM THERAPY FOR PATIENTS WITH
CORONARY ABNORMALITIES


Aspirin 3-5 mg/kg once daily orally



Clopidogrel 1 mg/kg/day (max 75 mg/day)



Most experts add warfarin or low-molecular-weight
heparin for those patients at particularly high risk of
thrombosis


ACUTE CORONARY THROMBOSIS


Fibrinolytic therapy with tissue plasminogen activator
Or



Other thrombolytic agent under supervision of a
pediatric cardiologist


Complication





Coronary artery aneurysm
Acute thrombosis
Coronary artery stenosis
Reyes syndrome


• Defined by persistent or recrudescent fever 36 hr
after completion of the initial IVIG infusion
• Another dose of IVIG at 2 g/kg is administered to
patients with IVIG resistance.
• Intravenous methylprednisolone
• If a second dose of IVIG or corticosteroids are
ineffective
• Cyclophosphamide and plasmapheresis.
• Tumor necrosis factor inhibitor infliximab


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Kawasaki disease

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